Kobayashi T, Ando Y, Umino T, Miyata Y, Muto S, Hironaka M, Asano Y, Kusano E
Department of Nephrology and Pathology, Jichi Medical School, Minamikawachi, Kawachi, Tochigi, Japan.
Clin Nephrol. 2006 Jun;65(6):423-6. doi: 10.5414/cnp65423.
We report a case of a 17-year-old male with relapse of minimal-change nephrotic syndrome (MCNS), in whom apheresis monotherapy without steroids or immunosuppressants resulted in complete remission. The patient initially developed nephrotic syndrome in February 1998. The first renal biopsy confirmed the diagnosis of MCNS. The patient was also found to be a carrier of hepatitis B virus. Steroid therapy was started with oral prednisolone 60 mg/day. Complete remission was achieved in 3 months, and the steroid treatment was tapered off in May 2001. During the steroid tapering, temporal exacerbation of liver function was noted. In July 2002, the patient was admitted to our hospital again due to relapse of nephrotic syndrome. Second biopsy reconfirmed the diagnosis of MCNS. Since the serum titer of HBV was elevated, apheresis monotherapy was selected to avoid the risk of steroid-induced fulminant hepatitis. Four sessions of low-density lipoprotein apheresis (LDL-A) and 5 sessions of double-filtration plasmapheresis (DFPP) reduced the proteinuria from 9.2 g/day to 0.2 g/day over 38 days without any additional medication. Proteinuria remained suppressed below 0.2 g/day for more than 12 months and no exacerbation of liver function was observed up to the final follow-up in September 2003. The present case suggested the potential of apheresis monotherapy to induce and maintain complete remission of MCNS and an important role of circulating factors in the pathogenesis of MCNS.
我们报告一例17岁男性微小病变型肾病综合征(MCNS)复发患者,其采用不使用类固醇或免疫抑制剂的单采疗法实现了完全缓解。该患者于1998年2月首次出现肾病综合征。首次肾活检确诊为MCNS。该患者还被发现是乙肝病毒携带者。开始使用口服泼尼松龙60mg/天进行类固醇治疗。3个月内实现完全缓解,2001年5月逐渐减少类固醇治疗。在逐渐减少类固醇剂量期间,注意到肝功能暂时恶化。2002年7月,患者因肾病综合征复发再次入住我院。第二次活检再次确诊为MCNS。由于乙肝病毒血清滴度升高,选择单采疗法以避免类固醇诱导的暴发性肝炎风险。在38天内进行了4次低密度脂蛋白单采(LDL-A)和5次双重滤过血浆置换(DFPP),无需任何额外药物治疗,蛋白尿从9.2g/天降至0.2g/天。蛋白尿在12个月以上一直维持在0.2g/天以下,直至2003年9月最后一次随访时未观察到肝功能恶化。本病例提示单采疗法诱导和维持MCNS完全缓解的潜力以及循环因子在MCNS发病机制中的重要作用。